Atypical inflammatory demyelinating syndromes of the CNS

Todd A. Hardy, Stephen W. Reddel, Michael H. Barnett, Jacqueline Palace, Claudia F. Lucchinetti, Brian G. Weinshenker

Research output: Contribution to journalReview articlepeer-review

60 Scopus citations


Atypical inflammatory demyelinating syndromes are rare disorders that differ from multiple sclerosis owing to unusual clinical or MRI findings or poor response to treatments used for multiple sclerosis. These syndromes include neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, Baló's concentric sclerosis, Schilder's disease, and Marburg's multiple sclerosis. The overlapping features of these syndromes with multiple sclerosis and with each other complicate diagnosis and their categorisation as distinct or related conditions. Recognition of these syndromes is crucial because they differ from multiple sclerosis and other demyelinating and non-demyelinating conditions in their prognosis and treatment. Advances in MRI, pathology, and immunobiology are needed to increase understanding of these syndromes, including the extent to which some of them represent distinct entities, and to assist with improvements in their diagnosis and management.

Original languageEnglish (US)
Pages (from-to)967-981
Number of pages15
JournalThe Lancet Neurology
Issue number9
StatePublished - Aug 1 2016

ASJC Scopus subject areas

  • Clinical Neurology


Dive into the research topics of 'Atypical inflammatory demyelinating syndromes of the CNS'. Together they form a unique fingerprint.

Cite this